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Management of Disorders of the Ductal System and Infections

CHAPTER CONTENTS

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Disorders of the ductal system can present as nipple discharge, nipple inversion, a breast mass, or periareolar infection.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

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Nipple discharge accounts for approximately 5% of refer- rals to breast clinics. It is a frightening symptom because of the fear of breast cancer. Approximately 95% of women pre- senting to the hospital with nipple discharge have a benign cause for the discharge. Discharge associated with a signifi- cant underlying pathologic process is spontaneous and more likely to be unilateral, arise from a single duct, be persistent (defined as more than twice per week), be troublesome, and be bloodstained or contain blood on testing. One study of 416 women with discharge identified bloody nipple discharge (odds ratio 3.7) and spontaneous discharge (odds ratio 3.2) as significant factors associated with a causative lesion (1).

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

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For this reason, the physician must establish whether the discharge is spontaneous or induced, whether it arises from a single or from multiple ducts, and whether it is from one or both breasts. The characteristics of the discharge also need to be defined: whether it is serous, serosanguineous, bloody, clear, milky, green, or blue-black. The frequency of discharge and the amount of fluid also need to be assessed; this assessment is important for milky discharge, as galac- torrhea should be diagnosed only if the milky discharge is spontaneous, copious in amount, and arises from multiple ducts of both breasts.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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Assessment should include the performance of a complete physical examination (Chapter 4) to identify the presence or absence of a breast mass. During the examination, firm

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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pressure should be applied around the areola as pressure over a dilated duct will often produce the discharge; this is helpful in defining where an incision should be made for any subsequent surgery. The nipple is squeezed with firm digi- tal pressure and, if fluid is expressed, the site and charac- ter of the discharge are recorded. Testing the discharge for hemoglobin determines whether blood is present. Bloody discharge increases the risks of cancer being the cause for the discharge with an odds ratio (OR) 2.27, 95% confidence intervals (CI) 1.32–3.89, p < .001. In a recent meta-analysis, up to 20% of patients who had a bloodstained discharge or who had a discharge containing moderate or large amounts of blood had an underlying malignancy (2). The absence of blood in nipple discharge is not an absolute indication that the discharge is not related to an underlying malignancy; in one series of 108 patients the sensitivity of hemoccult testing was only 50% (3). If the discharge is serous or col- ored but spontaneous and persistent, then malignancy still needs to be excluded. Age is said to be an important pre- dictor of malignancy; in one series, 3% of patients younger than 40 years of age, 10% of patients between ages 40 and 60 years, and 32% of patients older than 60 years who pre- sented with nipple discharge as their only symptom were found to have cancer. Cytology of nipple discharge is of little value in determining whether duct excision should be per- formed. In a recent study of 618 patients who had nipple discharge cytology, the sensitivity and specificity of cytol- ogy were 16.7% and 66.1%, respectively. In comparison, the sensitivity for macroscopically bloodstained discharge was 60.6% with a specificity at 53.6% (4). Although some studies have reported better results with cytology, the variability of reported results is such that it cannot be relied on in the routine assessment of nipple discharge.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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Two related techniques have emerged: ductal lavage,

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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in which fluid-yielding nipple ducts are cannulated at their

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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38

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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orifices and lavaged with saline while the breast is inter- mittently massaged (Chapter 20); and ductoscopy, in which discharging or fluid-yielding duct orifices are dilated and intubated with a microendoscope, and the lumen directly visualized. Both techniques have significant potential in terms of allowing repeated sampling of ductal epithelium over time and diagnosing the cause of nipple discharge (5). To learn ductoscopy takes longer than 6 months to over- come technical problems. Fiberoptic ductoscopy applied to 415 patients with nipple discharge was successful in identifying a lesion in 166 patients (40%) (6). Of these 166, 11 were subsequently shown to have ductal carcinoma in situ (DCIS); ductoscopy was suspicious in 8, a sensitivity of 73%, with a specificity of 99% and a positive predictive value of 80% (6). DCIS in this series tended to affect more peripheral ducts compared with papillomas. Numerous other small series have evaluated ductoscopy in nipple dis- charge (7,8). The sensitivity for malignancy in these other series varies from 81% to 100% (8). Ductoscopy appears of particular value for directing duct excision (7) and for detecting deeper lesions that can be missed by blind cen- tral duct excision (8). Surgical resection of lesions visual- ized on ductoscopy is facilitated by transillumination of the skin overlying the lesion. Lesions visualized by duc- toscopy can be sampled; in one report, 38 of 46 women with biopsy-proved papillomas were observed for 2 years with no case of missed cancer becoming evident (8). Newer biopsy devices using vacuum assistance are now available for diagnostic assessment and can be ductoscope or sono- graph guided.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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Ductal lavage increases cell yield approximately 100

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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times compared with analysis of discharge alone, aver- aging 5,000 cells per washed duct in one series (6). The sensitivity for cytology obtained by ductal lavage in this series was 64%, with a 100% positive predictive value. Other studies have reported lower sensitivities in the range of 50%, but a high specificity and a high overall accuracy rate (5). Both ductoscopy and ductal lavage remain investigative techniques, and the evidence that they are valuable in the detection of significant breast disease is limited.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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Imaging of the ductal tree by ductography or galactog- raphy can identify intraductal lesions. Although this inves- tigation has only a 60% sensitivity for malignancy, a filling defect or duct cutoff has a high positive predictive value for the presence of either a papilloma or a carcinoma (9). In one report, ductography-directed excisions were significantly more likely than central duct excisions to identify a spe- cific underlying lesion (10). Ductography in one large study was, however, a poor predictor of underlying pathology and could not exclude malignancy (11). The value of ductogra- phy is that like ductoscopy, it can allow identification of the site of any lesion in younger women, allowing localization and excision of the causative lesion while retaining the abil- ity to lactate.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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Mammography has a high overall sensitivity for breast cancer, but not all malignant lesions that cause nipple dis- charge are visible mammographically and most patients with nipple discharge have negative mammograms (Chapter 12). In one series, the sensitivity of mammogra- phy for malignancy in patients with nipple discharge was only 57% with a positive predictive value of 16.7% and a negative predictive value of 91.4% (3). Nonetheless, mam- mography should be performed in women of appropriate age, because if a lesion is visualized it may help establish the cause of the discharge. Ultrasound has a low sensitiv- ity for malignancy in patients with nipple discharge but is a valuable method for localizing intraductal abnormalities,

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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especially papillomatous lesions, in patients with no other clinical or radiologic findings (12). Any lesion visualized can be biopsied by core biopsy or excised using a vacuum- assisted large core biopsy device. (10,13) Patients with a visible lesion on ultrasonography appear significantly more likely to have malignancy than those women with a negative scan (10).

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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Is bloodstained or contains moderate or large amounts of blood on testing

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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Is persistent and stains clothes (occurs on at least two occasions per week)

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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Is associated with a mass

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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Discharge from multiple ducts normally requires surgery only when it causes distressing symptoms, such as persis- tent staining of clothes. Some breast units adopt an age- related policy: Patients younger than age 30 years who have serous, serosanguineous, or watery discharge are observed, with microdochectomy reserved for cases in which dis- charge persists at review; patients older than 45 years of age are treated by a formal excision of the major duct sys- tem on the affected side; patients between 30 and 45 years of age are deemed suitable for either approach. The current evidence is that total duct excision is more effective than microdochectomy at establishing a specific diagnosis and has a lower chance of missing any underlying malignancy in women more than 40 years of age (15). Today, many units incorporate ductography and ductoscopy into their man- agement protocols, particularly in younger women (Fig. 5-1). The problem is how to treat a patient with nipple discharge in whom imaging, including ductography or ductoscopy and ductal lavage, fails to identify any serious lesion. Some argue that as discharge from malignant disease is more likely to be bloodstained, there is no place for conservative manage- ment of bloodstained discharge and that all patients with bloodstained discharge should undergo duct excision unless investigation has identified a specific benign cause (16). Others argue that in selected patients, who have no clinical

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Investigations

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FIGURE 5-1 Investigation of nipple discharge.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Differential Diagnosis of Nipple Discharge

Physiologic Causes nan

In two-thirds of nonlactating women, a small quantity of fluid can be expressed from the ducts of the nipple if the nipple is cleaned, the breast massaged, and pressure applied. This fluid is physiologic secretion and varies in color from white to yellow to green to brown to blue-black; it is thought to represent apocrine secretion, as the breast is a modified apocrine gland. This physiologic secretion usually emanates from multiple ducts, and the discharge from each duct can vary in color. It is commonly found after pregnancy and is often noticed after a warm bath or after nipple manipulation. The discharge is not usually spontaneous or bloodstained and no specific treatment is required.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Differential Diagnosis of Nipple Discharge

Intraductal Papilloma nan

A true intraductal papilloma develops in one of the major subareolar ducts and is the most common lesion causing a serous or bloody nipple discharge. In approximately half of women with papillomas, the discharge is bloody; in the other half, it is serous (9). Papillomas should be differen- tiated from papillary hyperplasia, which affects the termi- nal duct lobular unit and can also cause nipple discharge. Central papillomas consist of epithelium covering arbores- cent fronds of fibrovascular stroma attached to the wall of the duct by a stalk (Fig. 5-2). The covering epithelium has a two-cell population, with a cuboidal or columnar cell lining covering an underlying layer of myoepithelial cells. A mass may be felt on examination in as many as one-third of cases.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Differential Diagnosis of Nipple Discharge

Intraductal Papilloma nan

Occasionally, the papilloma is so close to the nipple that it can be seen in the orifice of the duct at the nipple. The treat- ment of choice is microdochectomy. A solitary papilloma is not thought to be a premalignant lesion and is considered by some to be an aberration rather than a true disease pro- cess. Papillary lesions can be difficult to characterize on core biopsies.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Differential Diagnosis of Nipple Discharge

Multiple Intraductal Papillomas nan

In approximately 10% of patients with intraductal papil- lomas, multiple lesions are found; usually, two or three occur, often in the same duct. The term multiple intraductal papilloma syndrome is reserved for the rare and distinctive group of patients in whom one duct system contains five or more large and often palpable papillomas with a periph- eral distribution. Nipple discharge is less common than in

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Differential Diagnosis of Nipple Discharge

Multiple Intraductal Papillomas nan

FIGURE 5-2 Histology of duct papilloma.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Differential Diagnosis of Nipple Discharge

Juvenile Papillomatosis nan

A rare condition, juvenile papillomatosis, affects women between the ages of 10 and 44 years (19). The common pre- sentation is nipple discharge +/ a discrete mass lesion. In one series of 13 patients, 11 had peripheral and 2 central lesions (19). Three of the 13 presented with nipple discharge; 2 had a palpable peripheral mass lesion, and the remainder had nipple discharge alone. Treatment is by complete exci- sion. Patients with this condition may be at some increased risk of subsequent breast cancer, and close clinical and radiological surveillance of any woman with this condition is indicated.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Differential Diagnosis of Nipple Discharge

Carcinoma nan

FIGURE 5-3 Ultrasound of an intraduct papilloma char- acteristic of those seen in multiple papilloma syndrome— such lesions can be excised by mammotomy.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Differential Diagnosis of Nipple Discharge

Bloody Nipple Discharge in Pregnancy nan

Nipple discharge with blood present, either visibly or cyto- logically, during pregnancy or lactation is common. In 20% of women who experience nipple discharge during preg- nancy, blood is evident clinically. The likely cause is hyper- vascularity of developing breast tissue; it is benign, usually settles quickly, and requires no specific treatment. Only if it persists is investigation required.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Differential Diagnosis of Nipple Discharge

Galactorrhea nan

by a history of galactorrhea, amenorrhea, and relative infertility. Galactorrhea disappears after appropriate drug therapy or surgical removal of any pituitary adenoma. Appropriate drug therapy includes administration of caber- goline. Bromocriptine is an alternative, but it is no longer used because it produces significant side effects in up to one-third of patients including, very rarely, strokes (24). For patients with troublesome galactorrhea who are intolerant of medication, bilateral total duct ligation is effective.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Periductal Mastitis and Duct Ectasia

Clinical Syndromes nan

Periductal mastitis is characterized clinically by episodes of periareolar inflammation with or without an associated mass, a periareolar abscess, or a mammary duct fistula. Nipple retraction can be seen early at the site of the affected duct and is often subtle. Nipple discharge can also occur and is often purulent.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Periductal Mastitis and Duct Ectasia

Clinical Syndromes nan

The clinical features of duct ectasia include nipple retrac- tion at the site of the shortened duct or ducts and creamy or cheesy, viscous, toothpaste-like nipple discharge. Patients with green discharge from multiple ducts are often diag- nosed as having duct ectasia, but most of these have leaking physiologic breast secretion. In one large series, periductal mastitis principally affected women between the ages of 18 and 48 years, whereas most patients who presented with duct ectasia were aged between 42 and 85 years.

Management of Disorders of the Ductal System and Infections

NIPPLE DISCHARGE

Etiology

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Etiologic data thus suggest that periductal mastitis and duct ectasia are separate conditions with different causes. Duct ectasia appears to be an involutionary phenomenon, whereas periductal mastitis is a disease in which smoking and bacteria are important causal factors.

Management of Disorders of the Ductal System and Infections

NIPPLE INVERSION OR RETRACTION

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FIGURE 5-4 Nipple adenomas.

Management of Disorders of the Ductal System and Infections

NIPPLE INVERSION OR RETRACTION

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FIGURE 5-5 Nipple inversion from breast cancer. FIGURE 5-6 Slit-like nipple retraction from duct ectasia.

Management of Disorders of the Ductal System and Infections

NIPPLE INVERSION OR RETRACTION

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older than 35 years, a mammogram. Management depends on the presence or absence of a clinical or mammographic abnormality (Fig. 5-7). Central, symmetric, transverse slit-like retraction is characteristic of benign disease; nipple inver- sion occurring in association with either breast cancer or inflammatory breast disease is more likely to involve the whole of the nipple and, in a breast cancer, to be associated with distortion of the areola, which may be evident only when the breast is examined in different positions (Figs. 5-5 and 5-6). Benign nipple retraction requires no specific treatment, but can be corrected surgically if the patient requests it and the surgeon considers the operation appropriate. Division or excision of the underlying breast ducts (total duct division or excision) may be required to evert the nipple; patients should be warned that they will not be able to breast-feed after this procedure and may lose some nipple sensation.

Management of Disorders of the Ductal System and Infections

OPERATIONS FOR NIPPLE DISCHARGE OR RETRACTION

Microdochectomy

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necessary after this procedure, any significant defect can be closed with mobilization of adjacent breast tissue, and the skin is closed in layers with absorbable sutures. Papillomas visible on ultrasonography can be removed by needle local- ization or percutaneous vacuum-assisted biopsy.

Management of Disorders of the Ductal System and Infections

OPERATIONS FOR NIPPLE DISCHARGE OR RETRACTION

Total Duct Excision or Division

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Total duct excision can be a diagnostic procedure in older patients with nipple discharge and is indicated for multiple troublesome duct discharge or nipple eversion, and as treat- ment for periductal mastitis and its associated complica- tions. For nipple eversion duct division may be all that is required. Because the lesions of periductal mastitis usually contain organisms (Table 5-1), patients having operations for this condition should receive appropriate perioperative antibiotic treatment. Options for antibiotic therapy include amoxicillin–clavulanate or a combination of erythromycin and metronidazole hydrochloride. Some surgeons prefer total duct excision in older women with single-duct dis- charge who no longer wish to breast-feed. The reasoning is that is it is more likely than single-duct excision to obtain a specific diagnosis (15,16) and if there is a condition, such as duct ectasia, that affects all the ducts underneath the nipple, then any further discharge from the other affected ducts will be prevented. A circumareolar incision based at the six o’clock position is used unless a previous scar exists, in which case the same scar is reused. Dissection is per- formed under the areola down either side of the major ducts. Curved tissue forceps are passed around the ducts, and these are delivered into the wound. The ducts are secured and then divided from the undersurface of the nipple and, if a total duct excision is being performed, a 2- to 5-cm portion of ducts is excised depending on whether the operation is diagnostic or therapeutic.

Management of Disorders of the Ductal System and Infections

OPERATIONS FOR NIPPLE DISCHARGE OR RETRACTION

Total Duct Excision or Division

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For patients having cosmetic nipple eversion, the pro-

Management of Disorders of the Ductal System and Infections

OPERATIONS FOR NIPPLE DISCHARGE OR RETRACTION

Total Duct Excision or Division

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digital pressure to stretch the tissue stopping the nipple from everting; only rarely are sutures required under the nipple to maintain nipple eversion. No drains are placed, and the wound is closed in layers with absorbable sutures. Patients should be warned before surgery that this opera- tion results in significantly reduced nipple sensitivity in up to 40% of women.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

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Breast infection presenting to surgeons is much less com- mon clinically now than it was previously because of early use of antibiotics in the community. It is occasionally seen in neonates, but most commonly affects women between the ages of 18 and 50 years. In the adult, breast infection can be considered lactational or nonlactational. Infection can also affect the skin overlying the breast, and occurs either as a primary event or secondary to a lesion in the skin, such as an epidermoid cyst, or a more generalized condition, such as hidradenitis suppurativa. The organisms responsible for different types of breast infection and the most appropriate antibiotics with activity against these organisms are sum- marized in Table 5-1 (29). The guiding principle in treating breast infection is to give antibiotics as early as possible to stop abscess formation; if the infection or inflammation fails to resolve after one course of antibiotics, then abscess formation or an underlying cancer should be suspected (30).

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Mastitis Neonatorum

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Continued enlargement of the breast bud in the first week or two of life occurs in approximately 60% of newborns, and these enlarged buds can become infected, most often by Staphylococcus aureus, although the responsible organism is sometimes Escherichia coli. In the early stage, antibiotics (flucloxacillin) can control infection; however, if a localized collection is evident on ultrasound, incision and drainage, by aspiration or a small stab incision placed as peripherally as possible so as not to damage the breast bud, is effective at producing resolution.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Lactational Infection

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Lactational infection is now less common than it used to be. The infection is usually caused by S. aureus, but it can also be caused by S. epidermidis and Streptococcus species. The first stage is often development of a cracked nipple or a skin abra- sion due to nipple trauma from breast-feeding that results in both swelling, which compresses the subareolar breast ducts, and a break in the body’s defense mechanisms, which

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Lactational Infection

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Inflammatory cancers can be difficult to differentiate

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Lactational Infection

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from abscesses. If an abscess is evident on ultrasonography and the overlying skin is not thinned or necrotic, the abscess can be aspirated to dryness following injection of local anes- thesia into the skin and the breast tissue and the cavity irri- gated with local anesthetic to minimize pain and to dilute thick pus. The abscess should be irrigated until all the pus is evacuated and the fluid aspirated is clear. A combination of repeated aspiration and oral antibiotics is usually effective at resolving local abscess formation and is the current treat- ment of choice for most breast abscesses (29,30). Aspiration should be repeated every 2 to 3 days until no further pus is obtained. Characteristically, the fluid aspirated changes over a few days from pus to serous fluid and then to milk. If the skin overlying the abscess is thinned and pus is visible superficially on ultrasonography, then after application of local anesthetic cream or infiltration of local anesthetic into the overlying skin, a small incision (mini-incision) is made over the point of maximal fluctuation, and the pus is drained

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Lactational Infection

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(29). The cavity is then irrigated with local anesthetic solu- tion, which produces some pain relief. Irrigation is contin- ued every few days until the incision site closes. If the skin overlying the abscess is clearly necrotic, the necrotic skin can be excised to allow the pus to drain.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Lactational Infection

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FIGURE 5-8 (A) Lactational breast infection: large abscess was present on ultrasound which was treated by aspiration with rapid resolution (B).

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Lactational Infection

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FIGURE 5-9 Ultrasound of an abscess.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Nonlactational Infection

Periareolar Infection nan

FIGURE 5-10 Periareolar abscess with skin necrosis: the abscess can be drained by excision of the necrotic skin.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Nonlactational Infection

Periareolar Infection nan

removal of all the affected ducts by total duct excision. This operation to remove all the subareolar ducts up to the nip- ple skin is usually curative. Rarely subareolar abscesses can be caused by actinomyces species; these resolve following incision and drainage (32).

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Nonlactational Infection

Mammary Duct Fistula nan

FIGURE 5-11 Mammary duct fistula. Bilateral mammary duct fistula. On each side the fistula is discharging in the periareolar region. The affected duct is pulled toward the fistula.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Nonlactational Infection

Mammary Duct Fistula nan

Peripheral Nonlactational Breast Abscess Peripheral nonlactational breast abscesses are less common than periareolar abscesses and have been reported to be associated with a variety of underlying disease states, such as diabetes, rheumatoid arthritis, steroid treatment, and trauma. S. aureus is the organism usually responsible, but some abscesses contain anaerobic organisms. Peripheral nonlactational breast abscesses are three times more com- mon in premenopausal women than in menopausal or post- menopausal women and in most no obvious underlying cause is evident; following resolution of infection, mammog- raphy is indicated in women older than 35 years to exclude any underlying comedo DCIS. Systemic evidence of malaise and fever is usually absent. Management is the same as for other breast abscesses, with aspiration or incision and drainage (Fig. 5-12).

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Skin-Associated Infection

Cellulitis nan

Cellulitis is an uncommon infection in the breast and can be difficult to distinguish from inflammatory breast cancer or benign erythematous conditions of the breast (Fig. 5-13). Pain is a prominent feature of breast cellulitis associated with erythema, swelling, and warmth. Treatment is with antibiotics (Table 5-1).

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Skin-Associated Infection

Eczema nan

Patients with eczema involving the skin overlying the breast may develop secondary cellulitis. Appropriate treatments for eczema reduce the likelihood of recurrence.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Skin-Associated Infection

Hidradenitis Suppurativa nan

Hidradenitis suppurativa is a condition that affects the apocrine sweat glands and can result in recurrent infection and abscess formation of the skin of the lower half of the breast as well as the axilla (29,32,34–36). It is more common in smokers. Treatment involves keeping the area of skin as clean and dry as possible, draining any abscesses, and stop- ping smoking. A variety of drug treatments have been tried but are only partially effective. Excision and skin grafting of the affected skin has been tried and has a success rate of up to 50%.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Skin-Associated Infection

Intertrigo nan

Intertrigo is inflamed skin in the inframammary folds, often due to moisture and maceration (37) (Fig. 5-14). This can be a recurrent problem in women with large ptotic breasts that make contact with the chest wall. Fungi play

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Skin-Associated Infection

Intertrigo nan

FIGURE 5-12 (A) Peripheral abscess: note the shiny thin skin. This abscess was treated by min-incision and drainage with resolution (B).

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Skin-Associated Infection

Piercing nan

Nipple rings can result in subareolar breast abscess and recurrent nipple infections, particularly in smokers (38). One study noted that nipple piercing was a significant risk factor for a subareolar breast abscess (OR 10.2 95% CI 1.3– 454.4) as is smoking (OR 8.0 95% CI 3.4–19.4) (38).

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Skin-Associated Infection

Pilonidal Sinuses nan

Pilonidal sinuses affecting the nipple have been reported in hair stylists and sheep shearers because loose hairs penetrate the skin and can result in inflammation and infection (29).

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

nan nan

Tuberculosis is rare in Western countries. The breast can be the primary site, but tuberculosis more commonly reaches the breast through lymphatic spread from axillary, mediastinal, or cervical nodes or directly from underlying structures, such as the ribs. Tuberculosis predominantly affects women in the lat- ter part of their childbearing years. An axillary or breast sinus is present in up to 50% of patients. The most common presen- tation is that of an acute abscess resulting from infection of an area of tuberculosis by pyogenic organisms (29,30). Treatment is with local surgery and antitubercular drug therapy.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

nan nan

Primary actinomycosis (32), syphilis, mycotic, helmin- thic, and viral infections occasionally affect the breast, but are rare. Actinomycosis organisms can be seen in hidradenitis. Molluscum contagiosum can affect the areola and present as wart-like lesions.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Granulomatous Lobular Mastitis nan

FIGURE 5-13 Cellulitis of the breast. FIGURE 5-14 Intertrigo pre and post.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Granulomatous Lobular Mastitis nan

FIGURE 5-15 Granulomatous lobular mastitis at presentation (A) and following resolution (B).

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Granulomatous Lobular Mastitis nan

frequently affected, but not all women with this condition are parous. In contrast to periductal mastitis, it is common in Asian rather than white women and few are smokers. This condition has recently been reported to be associated with hyperprolactinemia (including drug-induced) (39). Prolactin can contribute to a wide variety of physiological and pathologi- cal granulomatous cutaneous lesions, and it may do the same in the breast. The frequency of hyperprolactinaemia in women with granulomatosis lobular mastitis is not well documented, so the relevance of this observation is not clear. Rare reported causes of granulomatous mastitis include alpha-1 antitrypsin deficiency and Wegener’s granulomatosis. The role of organ- isms in the etiology of this condition is unclear. One study did isolate corynebacteria from 9 of 12 women with granulomatous lobular mastitis (40). The most common species isolated was the newly described Corynebacterium kroppenstedtii, followed by C. amycolatum and C. tuberculostearicum. These organisms are usually sensitive to penicillin and tetracycline and when antibiotics effective against these organisms have been admin- istered to patients with this condition they do not produce resolution. Any antibiotic treatment should therefore be based on sensitivities as reported by the local bacteriologic service.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Granulomatous Lobular Mastitis nan

A search for the etiology of this condition continues. In patients presenting with a breast mass diagnosed on core biopsy as granulomatous lobular mastitis, excision of the mass should be avoided because it is often followed by per- sistent wound discharge and failure of the wound to heal. Current treatment involves establishing the diagnosis and observation without any specific treatment because the condition usually resolves slowly over 6 to 12 months. Any abscesses that develop require aspiration or mini-incision and drainage. There is a strong tendency for this condition to recur, but eventually it does resolve spontaneously with- out treatment (29). Steroids have been tried but without consistent success. More recently, methotrexate as mono- therapy given at a dose of 7.5 mg per week, has been claimed to be effective (41). Similar claims were made for steroids. Whether methotrexate alters the course of the condition or merely suppresses the inflammatory component is not clear and given that the condition does resolve spontaneously more studies are required before methotrexate can be con- sidered as an effective therapy for this condition.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Breast Infection after Breast Surgery nan

Rates of infection after breast surgery vary in relation to the extent of the surgery and risk factors including smoking, obe- sity, and the presence of diabetes. Rates of infection in excess of 10% are seen after mastectomy (42). Preoperative antibiot- ics reduce the risk of breast infection by 36% therefore pre- operative prophylactic antibiotics in breast surgery patients may be administered routinely. The relative risk of infection if antibiotics are administered in a recent meta-analysis was 0.64, 95% confidence intervals 0.50–0.83, p < .0005 (43).

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Morrogh M, Park A, Elkin EB, et al. Lessons learned from 416 cases of nipple discharge of the breast. Am J Surg 2010;200(1):73–80.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Chen L, Zhou WB, Zhao Y, et al. Bloody nipple discharge is a predictor of breast cancer risk: a meta-analysis. Breast Cancer Res Treat 2012;132:9–14.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Kooistra BW, Wauters C, van de Ven S, et al. The diagnostic value of nipple discharge cytology in 618 consecutive patients. Eur J Surg Oncol 2009;35(6):573–577.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Khan SA, Baird C, Staradub VL, et al. Ductal lavage and ductoscopy: the opportunities and the limitations. Clin Breast Cancer 2002;3:185.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Dietz JR, Crowe JP, Grundfest S, et al. Directed duct excision by using mammary ductoscopy in patients with pathologic nipple discharge. Surgery 2002;132:582.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Matsunaga T, Ohta D, Misaka T, et al. Mammary ductoscopy for diag- nosis and treatment of intraductal lesions of the breast. Breast Cancer 2001;8:213–221.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Van Zee KJ, Ortega Perez G, Minnard E, et al. Preoperative galactography increases the diagnostic yield of major duct excision for nipple discharge. Cancer 1998;82:1874.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Cabioglu N, Hunt KK, Singletary SE, et al. Surgical decision making and factors determining a diagnosis of breast carcinoma in women presenting with nipple discharge. J Am Coll Surg 2003;196:354.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Rissanen T, Reinikainen H, Apaja-Sarkkinen M. Breast sonography in local- izing the cause of nipple discharge: comparison with galactography in 52 patients. J Ultrasound Med 2007;26(8):1031–1039.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Ko KH, Jung HK, Youk JH, et al. Potential application of ultrasound-guided vacuum-assisted excision (US-VAE) for well-selected intraductal papil- lomas of the breast: single-institutional experiences. Ann Surg Oncol 2012;19(3):908–913.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Foulkes RE, Heard G, Boyce T, et al. Duct excision is still necessary to rule out breast cancer in patients presenting with spontaneous bloodstained nipple discharge. Int J Breast Cancer 2011;495315.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Page DL, Anderson TJ. Diagnostic histopathology of the breast. Edinburgh, UK: Churchill Livingstone 1987.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Bazzocchi F, Santini D, Martinelli G, et al. Juvenile papillomatosis (epithe- liosis) of the breast: a clinical and pathologic study of 13 cases. Am J Clin Pathol 1986;86:745.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Obedian E, Haffty BG. Breast conserving therapy in breast cancer patients presenting with nipple discharge. Int J Radiat Oncol Biol Phys 2000;47:137.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Kato M, Oda K, Kubota T, et al. Non-palpable and non-invasive ductal carcinoma with bloody nipple discharge successfully resected after can- cer spread was accurately diagnosed with three-dimensional computer tomography and galactography. Breast Cancer 2006;13:360.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Fitzal F, Mittlboeck M, Trischler H, et al. Breast-conserving therapy for centrally located breast cancer. Ann Surg 2008;247:470.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Wang AT, Mullan RJ, Lane MA, et al. Treatment of hyperprolactinemia: a systematic review and meta-analysis. Syst Rev 2012;1(1):33.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Bundred NJ. Surgical management of periductal mastitis. Breast 1988;7:79.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Oyama T, Koerner FC. Noninvasive papillary proliferations. Semin Diagn Pathol 2004;21:32–41.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Hughes LE, Mansel RE, Webster DJT. Infections of the breast. In: Hughes LE, Mansel RE, Webster DJT, eds. Benign disorders and diseases of the breast: concepts and clinical management. 3rd ed. London: WB Saunders 2009:187.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Dixon JM, Thomas J. Congenital problems and aberrations of normal development and involution. In: Dixon JM, ed. ABC of breast diseases. 4th ed. London: Wiley-Blackwell 2012:12.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Attar KH, Waghorn D, Lyons M, et al. Rare species of actinomyces as caus- ative pathogens in breast abscess. Breast J 2007;13:501–505.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Atkins HJB. Mammillary fistula. BMJ 1955;2:1473.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol 2009;60:539.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

2009;96:350.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Janniger CK, Schwartz RA, Szepietowski JC, et al. Intertrigo and common secondary skin infections. Am Fam Physician 2005;72:833.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Gollapalli V, Liao J, Dudakovic A, et al. Risk factors for development and recurrence of primary breast abscesses. J Am Coll Surg 2010;211:41.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Lin CH, Hsu CW, Tsao TY, et al. Idiopathic granulomatous mastitis associ- ated with risperidone-induced hyperprolactinemia. Diagn Pathol 2012;7:2.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Akbulut S, Yilmaz D, Bakir S. Methotrexate in the management of idio- pathic granulomatous mastitis: review of 108 published cases and reports of four cases. Breast J 2011;17(6):661–668.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Olsen MA, Chu-Ongsakul S, Brandt KE, et al. Hospital-associated costs due to surgical site infection after breast surgery. Arch Surg 2008;143(1):53–60.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Sajid MS, Hutson K, Akhter N, et al. An updated meta-analysis on the effec- tiveness of preoperative prophylactic antibiotics in patients undergoing breast surgical procedures. Breast J 2012;18(4):312–317.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

Breast pain is one of the most common problems for which patients consult primary care physicians, gynecologists, and breast specialists. Patients mistakenly think the symptom is associated with early breast cancer, but data do not sup- port any strong relationship with breast pain. The Women’s Health Initiative Estrogen plus Progestin intervention trials showed no effect on breast cancer risk in women who took estrogens alone, but a mild effect in those taking equine estrogen plus medroxyprogesterone, particularly if baseline breast tenderness was present (hazard ratio [HR] 2.16), but the effect was much less if no baseline breast tenderness was present (1). Once cancer has been ruled out, reassurance alone will resolve the problem in 86% of those with mild and 52% of those with severe mastalgia (2). A survey of screened women in the UK national program revealed that 69% had experienced severe breast pain, although only 3% had sought treatment. Ader et al. in 2001 attempted to establish the prev- alence in the community in the United States. In their study, 874 women between 18 and 44 were recruited for interview by random number dialing in Virginia, and 68% reported some cyclical mastalgia, with 22% describing it as moderate or severe (3). Interestingly, patients on the oral contracep- tive pill had less trouble, while there was a positive asso- ciation with smoking, caffeine intake, and perceived stress. A study from the United States (4) showed the impact of breast pain among a population of 1,171 women attending a general obstetrics and gynaecology clinic. Sixty-nine percent suffered regular discomfort and 36% had consulted about their breast pain. A specialist breast clinic in Ghana reported in 2008 that 72% of women attended because of breast pain. Reading of the literature might suggest that the incidence of breast pain is different in many parts of the world, but these differences are mainly cultural in relation to the willingness of women to consult their physicians about breast pain.

Management of Disorders of the Ductal System and Infections

BREAST INFECTION

Other Rare Infections

Factitial Disease nan

The major clinical issue is to exclude cancer and deter-

Management of Disorders of the Ductal System and Infections

ETIOLOGY

nan nan

Breast swelling is a frequent event in the late luteal phase of the menstrual cycle. Cyclic mastalgia is a more extreme form of this change, and researchers have sought endocrine abnormalities in those with severe breast pain, particu- larly measuring estradiol, progesterone, and prolactin, but no major abnormalities have been found (5). One hypoth- esis suggested that inadequate corpus luteal function is an etiologic factor in women with benign breast disease, but this term has been used to include all nonmalignant breast conditions, blurring the distinction between a vari- ety of benign breast conditions. No evidence of proges- terone deficiency has been found during the luteal phase in patients with mastalgia. The confusion in the literature between the symptom of breast pain and the large num- ber of variable pathological descriptions of benign breast conditions has resulted in the belief that the condition is a “disease,” rather than physiological responses to men- strual cycles. In the aberrations of normal development and involution (ANDI) classification of benign conditions, mastalgia is regarded as a physiologic disorder arising from hormonal activity with little connection to cancer risk, or true pathologic conditions (6). Another suitable term might be benign breast change as this does not suggest cancer or premalignancy.

Management of Disorders of the Ductal System and Infections

ETIOLOGY

nan nan

in women with cyclic mastalgia; both normal levels and elevated levels have been reported during the luteal phase. Baseline levels of prolactin are either normal or marginally elevated, but increased prolactin release was found after domperidone stimulation in severe cyclic mastalgia, possi- bly representing a stress response to prolonged pain.

Management of Disorders of the Ductal System and Infections

ETIOLOGY

nan nan

Ecochard et al. measured a range of personal and endo- crine variables in 30 women with mastalgia and 70 control subjects (7). Cases were more likely to report foot swelling or abdominal bloating (43% vs. 19%). Women with mastalgia had higher mean luteal levels of luteinizing hormone (LH) and follicle-stimulating hormone (FSH).